Pyodermatitis vegetans (PDV) is a rare inflammatory disorder that is often associated with underlying inflammatory bowel disease (IBD), particularly ulcerative colitis, and less commonly with primary sclerosing cholangitis. Men are more frequently affected than women, and patients are usually aged 20-60 years when diagnosed. The onset of bowel disease can precede this disease by years. PDV may also be a marker of subclinical disease. Most patients with PDV develop mucosal lesions as well. The term pyostomatitis-pyodermatitis vegetans (PSV-PDV) is used in cases of mucocutaneous disease. The pathogenesis of PSV and PDV is poorly understood.